A 30-year-old male who had recently been treated for asthma exacerbation with steroid therapy presents to the vascular medicine clinic for follow up 4 weeks after visiting the emergency department (ED) with complaints of hearing issues with tinnitus, vertigo, and headaches. Over the preceding weeks, he began to develop nonspecific features of fever, fatigue, and mild weight loss. He noted slight improvement in tinnitus, vertigo, and headaches while on steroid therapy. He has been smoking one pack of cigarettes for 10 years and noted occasional skin changes when the weather is cold. He denied any proximal muscle pain or weakness. Pertinent physical exam findings include a blood pressure of 135/74 mmHg in the right arm and 133/70 mmHg in the left arm; a normal cardiac S1, S2 with a 1/6 diastolic decrescendo murmur best heard at the left sternal border with a Corrigan pulse. A soft bruit was appreciated in the mid-epigastrium. His pulse exam included the following: carotid and radial pulses were normal; the temporal arteries were non-tender; the lower extremities were normal. Pertinent laboratory evaluation in the ED was as follows: complete blood count was remarkable for low hemoglobin of 11.5 g/dL (normocytic anemia), a mild leukocytosis with a white blood count of 12.5 x 109/L, a normal platelet count of 329 x 109/L, and an elevated sedimentation rate of 87 mm/hr (normal <29 mm/hr) and C-reactive protein of 85 mg/L (normal <8 mg/L).
The correct answer is: D. Cogan's syndrome
Cogan's syndrome is an uncommon clinical disease which primarily affects young adults before the age of 40 years. The cause of Cogan's syndrome remains unknown but is associated more often with a large vessel vasculitis than medium sized arteries. This syndrome was first published in Archives of Ophthalmology in 1945 after D.G. Cogan, an ophthalmologist, described four patients having symptoms of hearing loss, vertigo, tinnitus, and ocular symptoms.1 Although it usually will begin with only one symptom, most patients will typically manifest with systemic, audiovestibular, ocular, and/or vascular symptoms (Table 1). Symptoms typically include interstitial keratitis, hearing loss, and systemic inflammation (Takayasu-like vasculitis) within 1 year of onset,2 Blindness occurs in about 5% of patients and if left untreated, most patients become deaf within 3 years.3 Aortitis can lead to aortic insufficiency in 15% of patients.4,5 The diagnosis requires the presence of inner ear dysfunction and the presence of eye inflammation that is not explained by other conditions. Laboratory testing may show signs of leukocytosis, elevated ESR and C-reactive protein, anemia (~1/3 of patients), and thrombocytosis.2 Several studies suggest an association with anti-neutrophil cytoplasmic antibodies (ANCA) and anti-endothelial antibodies.6 Immunosuppressive therapy with high dose corticosteroids (typically prednisone 1 mg/kg daily) has remained the treatment of choice and should be started early in the course of the illness.7 Methotrexate and steroid-sparing therapies (leflunomide, azathioprine, cyclophosphamide, and tacrolimus) have been shown to be effective in certain cases and should be considered if failing to respond to steroids within 7-10 days.2,7 The condition is fatal in less than 10% of patients, however methylprednisolone therapy should be considered, at 1 gram per day for 3 days, with concomitant cyclophosphamide among individuals who present with life-threatening situations. Those with end-stage hearing loss should be consider for cochlear implants. Cogan's syndrome is a chronic condition and currently there is no known way to prevent this disease.
Table 1: Characteristics of Cogan's Syndrome
| Constitutional |
Vestibular-Auditory |
Eye |
Vascular |
- Fever
- Headache
- Fatigue
- Night sweats
- Weight loss
- Arthralgia
- Poor balance
- Nausea
|
- Vertigo, ataxia
- Tinnitus
- Aural pressure
- Sensorineural hearing loss, which may become profound and permanent
- Ménière like episodes
|
- Bilateral Interstitial Keratitis
- Choroiditis
- Uveitis
- Vitreitis
- Retinal vasculitis
- Scleritis
- Episcleritis
- Pain, lacrimation
|
- Coronary arteritis
- Aortitis
- Aortic insufficiency
- Aortic and large arterial aneurysms
- Mesenteric vasculitis
|
(Answer A) Ascending aortic dissections are typically a life-threatening condition that occurs when a tear in the aortic intima allows blood to penetrate and split the media into two separate layers. Characteristic symptoms are acute onset of severe chest, neck, back or abdominal pain. The pain is typically abrupt and most severe at its onset, rather than the crescendo pattern that usually accompanies an acute coronary syndrome. On physical exam, patients may be acutely hypotensive, may have a blood pressure differential between the arms and/or a pulse deficit in the lower extremities, and may also have a diastolic murmur of aortic insufficiency. Risk factors for medial degeneration of the aorta include advanced age, bicuspid aortic valve, history of hypertension, male gender, and a known aortic aneurysm. The early mortality for type A dissection is 1% per hour; urgently replacing the vulnerable ascending aorta with an interposition tube graft is paramount.
(Answer B) Giant cell arteritis (GCA) is a common inflammatory condition that often affects people over the age of 50 years. Characteristic symptoms are headaches, jaw claudication, scalp tenderness, visual symptoms, carotidynia or limb claudication, most of which this clinical case presents. Additionally, markers of inflammation are often elevated. Treatment requires higher dosages of corticosteroids and urgent referral to a rheumatologist as blindness is a serious morbidity of this disease if left untreated. Relapse is common in GCA. Therefore, surveillance is important, as is monitoring for long-term complications of corticosteroid use, such as osteoporosis and gastrointestinal ulcers. In addition, there is an association between polymyalgia rheumatica (PMR) and GCA; 40–60% of patients with GCA have PMR symptoms at diagnosis while 16–21% of patients with PMR develop GCA.
(Answer C) Thromboangiitis obliterans (TAO), also known as Buerger's disease, is a nonatherosclerotic segmental vasculitis that affects the small- and medium-sized arteries and veins of the extremities. It is strongly associated with tobacco exposure and usual occurs in young male subjects. TAO is histologically characterized by segmental obliteration of involved vessels and thrombosis occurs early in medium sized arteries and superficial veins followed by recanalization early on. In the chronic phase of TAO, segmental obliteration can cause long-term complications, including minor and major amputations, even death. Cooper et al. from the Mayo Clinic have previously reported that the risk of major amputation was 11% at 5 years, 21% at 10 years and 23% at 20 years. In addition, TAO differs from many other types of vasculitis in that the usual immunologic markers, such as elevation of erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP), are usually normal. Survival is decreased in TAO patients when compared to age- and gender-matched United States population. Abstinence from smoking is the only definitive treatment to prevent disease progression.
References
- Cogan DG. Syndrome of nonsyphilitic interstitial keratitis and vestibuloauditory syndromes. AMA Arch Ophthalmol 1945;33:144-49.
- Singer O. Cogan and Behcet syndromes. Rheum Dis Clin North Am 2015;41:75-91.
- Oldenski R. Cogan syndrome: autoimmune-mediated audiovestibular symptoms and ocular inflammation. J Am Board Fam Pract 1993;6:577-81.
- Cochrane AD, Tatoulis J. Cogan's syndrome with aortitis, aortic regurgitation and aortic arch vessel stenosis. Ann Thorac Surg 1991;52:1166-67.
- Vollertsen RS, McDonald TJ, Younge BR, Banks PM, Stanson AW, Ilstrup DM. Cogan's syndrome:18 cases and a review of the literature. Mayo Clin Proc 1986;61:344-61.
- Ikeda M, Okazaki H, Minota S. Cogan's syndrome with antineutrophil cytoplasmic autoantibody. Ann Rheum Dis 2002;61:761-62.
- Gluth MB, Baratz KH, Matteson EL, Driscoll CLW. Cogan's syndrome: a retrospective review of 60 patients throughout a half century. Mayo Clin Proc 2006;81:483-88.
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